AB0902 LEFT VENTRICULAR SYSTOLIC DYSFUNCTION IS RARE BUT PROGNOSTICALLY IMPORTANT IN SYSTEMIC SCLEROSIS
Authors: J. Fairley, D. Hansen, S. Proudman, J. A. Sahhar, G. S. Ngian, J. Walker, L. Host, A. La Gerche, D. Prior, K. Morrisroe, W. Stevens, M. Nikpour, L. Ross
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Reduced left ventricular ejection fraction (LVEF) occurs in only around 5% of unselected SSc cohorts <sup>[1]</sup>. Subclinical LV systolic dysfunction (LVSD) defined by advanced echocardiographic measures including global longitudinal strain (GLS) may be more prevalent <sup>[2]</sup>. <h3>Objectives</h3> To quantify the frequency of LVSD in SSc as measured by LVEF and LV GLS, and its impact on survival and physical function. <h3>Methods</h3> Included participants in the Australian Scleroderma Cohort Study met ACR/EULAR criteria for SSc and had ≥1 echocardiographic LVEF measurement. Those with secondary causes of LVSD (ischaemia, valvular disease and PAH) were excluded. Chi squared tests, two sample t-tests or Kruskal Wallis tests were used for between group comparison as appropriate. Generalised estimating equations were used to model longitudinal data. Survival analyses were performed using Kaplan-Meier and Cox proportional hazard models. <h3>Results</h3> Of 1145 participants, 2.5% recorded a reduced of LVEF&lt;50% during follow up; 14% had borderline LVEF of ≥50% but &lt;55%; and 83% always a LVEF ≥55%. Only 0.6% ever had a LVEF≤40%. LVEF returned to normal in 66% of those who had recorded a LVEF&lt;50%. Compared to those with normal LVEF, those with reduced LVEF were similar in age, more frequently male (p=0.01), with dcSSc (p&lt;0.01), SSc renal crisis (p=0.02), synovitis (p=0.04) and higher inflammatory markers (p&lt;0.02). Those with reduced LVEF more commonly had myositis (p&lt;0.01), muscle atrophy (p=0.01) and proximal weakness (p&lt;0.01). Use of prednisolone (p=0.03), IVIG (p&lt;0.01), and cyclophosphamide (p&lt;0.01) were more common in those with reduced LVEF. Survival was worse in those with reduced LVEF (Figure 1). Reduced LVEF was associated with a 2.8-fold increased risk of death (95% CI 1.3-6.3, p=0.01: Table 1). Physical function and quality of life were worse in those with reduced LVEF. Multivariate analyses identified shorter six minute walk distance (p=0.01), higher Health Assessment Questionnaire Disability Index scores (p&lt;0.01) and lower Short Form 36 Physical Component Summary scores (p=0.02) in those with reduced LVEF. Participants with reduced LVEF were 3.9-times more likely to record WHO Class III/IV dyspnoea (95%CI 1.8-8.1, p&lt;0.01). LV GLS data were available for 90% of participants at one centre (n=219). Abnormal LV GLS (&gt; -17%) was seen in 30% of those with normal LVEF and 53% with borderline LVEF. LV GLS was lowest in those with reduced LVEF (-17.2%; IQR -14.9 – -18.0%) compared to those with normal (-19.5%; IQR -18.0 – -20.8%) or borderline LVEF (-18.3%; IQR -16.6 – -19.5%; p&lt;0.01). Worst recorded GLS was associated with increased mortality (HR 1.3, 95%CI 1.0-1.6, p=0.02), although this association was attenuated in multivariate analyses (HR 1.2, 95%CI 1.0-1.5, p=0.10). <h3>Conclusion</h3> After excluding secondary causes, reduced LVEF in SSc is rare. Despite the relative infrequency, LVEF&lt;50% is associated with a specific SSc phenotype, worse survival and physical function. Abnormal LV GLS is more prevalent in SSc, seen in 30-50% of those with persistent LVEF≥50%. Further data are required to define the threshold of LVSD in SSc, and to explore the utility of measures of subclinical LVSD such as LV GLS. <h3>References</h3> [1]Allanore Y, Meune C, Vonk MC, et al. Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis. <i>Ann Rheum Dis</i> 2010; <b>69</b>(1): 218-21. [2]Tennoe AH, Murbraech K, Andreassen JC, et al. Systolic Dysfunction in Systemic Sclerosis: Prevalence and Prognostic Implications. <i>ACR Open Rheumatol</i> 2019; <b>1</b>(4): 258-66. <h3>Acknowledgements</h3> ASCS is supported by Janssen, Scleroderma Australia, Scleroderma Victoria, Arthritis Australia, Musculoskeletal Australia, Australian Rheumatology Association, St. Vincent's Hospital Melbourne, GSK, Pfizer, BMS, Roche and Bayer. JLF holds a NHMRC Postgraduate Scholarship GNT2013842 &amp; Australian Government Research Training Program Scholarship. KM holds NHMRC Investigator Grant GTN1197169. LR holds Arthritis Australia ARA Victoria Fellowship. MN holds an NHMRC Investigator Grant (GNT1176538). <h3>Disclosure of Interests</h3> Jessica Fairley Speakers bureau: Boeringher-Ingelheim February 2023 - presenter in ILD meeting, Dylan Hansen: None declared, Susanna Proudman Speakers bureau: Boehringer Ingelheim, Consultant of: Boehringer Ingelheim, Jo-Anne Sahhar Speakers bureau: Boehringer Ingelheim, Gene-Siew Ngian: None declared, Jenny Walker Speakers bureau: Boehringer Ingelheim, Lauren Host: None declared, Andre La Gerche: None declared, David Prior Speakers bureau: Janssen-Cilag, Novartis, Kathleen Morrisroe: None declared, Wendy Stevens Consultant of: Boheringer Ingelheim - advisory board fees, Mandana Nikpour Speakers bureau: Janssen, Boehringer Ingelheim, GSK, Consultant of: Janssen, Boehringer Ingelheim, Astra Zeenca, GSK, Laura Ross: None declared.